scholarly journals Unusual presentation of extramedullary haematopoiesis in a young boy

2019 ◽  
Vol 12 (3) ◽  
pp. e227199
Author(s):  
Pramod Darole ◽  
Uma Sundar ◽  
Nilesh Kuchekar ◽  
Ajay Karre

Acute transverse myelopathy in a young person may be due to infection, postinfective or inflammatory demyelination, or vascular causes. Rarely, a completely reversible cause of acute transverse myelopathy may be seen, as described here in our case of transverse myelopathy due to extramedullary haematopoiesis (EMH). An 18-year-old man who had a history of a lone blood transfusion at age of 7 years presented with paraplegia. MRI showed multiple epidural space masses of EMH compressing the spinal cord. He was detected to have thalassaemia intermedia and was treated with blood transfusions, steroids and radiotherapy to the involved paraspinal areas. He recovered fully over 15 days and remained symptom free at 6 months.

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4295-4295
Author(s):  
Akihiro Takeshita ◽  
Miwa Adachi ◽  
Dae Won Kim ◽  
Kyou Sup Han ◽  
So Yong Kwon ◽  
...  

Abstract Backgrounds: Allo-immunization to blood cell antigens has been frequently reported among transfusion recipients with hematological diseases and cancer, as well as during pregnancy. The frequency of irregular erythrocyte antibodies (Abs) ranges from 1 to 2 % among hospitalized patients. However, the frequency varied according to the genetic diversity of the population. Several studies involving transfusion cases have been conducted in various countries including Japan (Watanabe et al, ASH 2009). However, there is currently little information about irregular erythrocyte Abs in many Asian populations. Here, we show updated data concerning variations of irregular erythrocyte Abs in patients with a history of blood transfusions between South Korea and Japan. Materials and Methods: In all, 21 institutions from South Korea and 59 from Japan participated in this study. We investigated methods employed for screening and identifying irregular erythrocyte Abs. The frequencies of irregular Abs to D, C, c, E, e, f, Ce, P1, M, N, S, s, Mia, Lea, Leb, Jka, Jkb, Jk3, Fya, Fyb, K, k, Kpa, Kpb, Jsa, Jsb, Dia, Dib, Lua, Lub, Xga and H were studied. If a case was analyzed multiple times, it was counted once. Multiple antibodies detected in the same patient were counted separately. The frequencies of irregular erythrocyte Abs on the basis of blood transfusion history were analyzed and compared between patients from South Korea and Japan. We partially selected irregular erythrocyte Abs detected in patients that were initially negative before receiving a transfusion. Among them, we compared the efficacy of detection methods between the indirect antiglobulin test (IAT) and the enzyme method at the first detection of the Abs. Results: In total, antibodies were detected in 16,438 patients (3,525 from South Korea; 12,913 from Japan). The female to male ratios of patients from the South Korean and Japanese institutions were 1.53 and 1.43, respectively. The number of patients with and without a history of previous transfusion was 1,146 and 2,100 in South Korea, 3,609 and 8,185 in Japan, respectively. Anti-E (1.4x, p<0.01 in South Korea; 1.7x, p<0.01 in Japan), anti-C (1.8x, p=0.05 and 2.3x, p<0.01, respectively) and anti-Jka (1.8x, p=0.01 and 5.8x, p<0.01, respectively) were frequently detected in patients who had received blood transfusions in either country. Anti-c was 1.8 times more frequently detected in patients who had received transfusions in South Korea, but there was no significant increase observed in patients who had received transfusions in Japan. Anti-D did not increase in patients who had received a transfusion in either country. Anti-c+E (2.1x in South Korea and 3.4x in Japan), anti-C+e (2.6x and 3.2x, respectively) and anti-E+Jka (9.2x and 6.3x, respectively) complex Abs increased in patients who had received a blood transfusion. Anti-E was newly detected in 341 patients after receiving a blood transfusion. In 40 cases, anti-E was detected earlier by the enzyme method than by the IAT method. In 147 cases, anti-E was detected only by the enzyme method; while in 4 cases detection was confirmed only by the IAT procedure. Anti-E was simultaneously detected in 154 cases by both methods. Conclusion: The data presented in this study was derived from a collaboration between South Korea and Japan on alloimmunity to erythrocyte antigens. The total number of positive cases was more than 16,000. Anti-E, anti-C, anti-Jka, anti-c+E and anti-C+e were frequently detected among patients with a history of previous blood transfusions. These results were similar between South Korean and Japanese patients. Anti-c was frequently detected in transfused patients from South Korea, but the number of positive cases did not change in Japanese patients who had undergone a transfusion. These observations might be explained by the ethnic difference in allo-immunity among patients or detection methods adopted in the various participating institutions. Further data, including details of the detection method, will be analyzed in future studies. Figure 1 Figure 1. Figure 2 Figure 2. Disclosures No relevant conflicts of interest to declare.


Author(s):  
Coleen Adams ◽  
Derek Armstrong

ABSTRACT:Twenty-three children with acute transverse myelopathy (ATM) are reviewed. Antecedent minor trauma or exercise was reported in 10 patients. Despite a positive history in 7 patients no preceding infection was documented. Two patients had a history of less severe ATM followed by recovery prior to a second more severe episode. The most common initial symptom was back pain and the most prominent clinical signs were weakness, sensory level and sphincter disturbances. Myelography and CT myelography at presentation was performed to exclude a compressive lesion. Spinal cord enlargement was demonstrated in 6 of 21 cases. Magnetic resonance imaging (MRI) of the spinal cord, performed in one patient, showed enlargement of the cord. Poor prognostic features were severity of weakness at the time of maximum deficit and a delayed onset of recovery. Maximum motor recovery occurred at a mean of 6½ months but did not occur in one patient until 1½ years. Normal or good outcome was obtained in 64%.


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4697-4697
Author(s):  
James R. Cerhan ◽  
Eric Engels ◽  
Wendy Cozen ◽  
Scott Davis ◽  
Richard K. Severson ◽  
...  

Abstract Background. The incidence of non-Hodgkin lymphoma (NHL) has increased dramatically since at least the 1950s, and only a fraction of this increase can be explained by established risk factors. During this timeframe, there has been a major increase in the use of blood transfusions, anesthesia, and invasive surgical procedures, all of which can impact immune function. Methods. We conducted a population-based case-control study from 1998–2000 using SEER cancer registries in Detroit, Iowa, Los Angeles and Seattle. NHL cases (N=759) were newly diagnosed, HIV-negative, and aged 20–74 years. Controls (N=589) were identified through random digit dialing (<65 years old) and Medicare files (age 65 years and older), and were frequency matched to cases on sex, age, race, and study site. Data on history of blood transfusions, anesthetics (general and regional), and surgeries (type, frequency, and age for 21 anatomic regions) >1 year before diagnosis (or date of enrollment for controls) were collected during in-person interviews. Unconditional logistic regression was used to estimate the odds ratio (OR) and 95% confidence intervals (CI), adjusted for the matching factors. NHL subtypes (follicular and diffuse) were designated according to SEER cancer registry pathology reports, and risk of each subtype was estimated using polychotomous logistic regression. Results. History of blood transfusion was weakly associated with increased risk of NHL (OR=1.26; 95% CI 0.91–1.73), and the elevated risk was specific to transfusions first given 5 to 29 years before diagnosis (OR=1.69; 95% CI 1.08–2.62). Risk was also specific to blood transfusions given for a medical indication (OR=2.09; 95% CI 1.03–4.26), while transfusions given for trauma, obstetric or surgical indications were not associated with risk. Exposure to general or regional anesthesia (OR=1.35 for 24+ times compared to 0–6; 95% CI 0.91–2.02) and total number of surgeries (OR=1.22 for 7+ surgeries compared to 0; 95% CI 0.77–1.93) were weakly and positively associated with risk of NHL, although neither association achieved statistical significance. Results were similar for general versus regional anesthesia. In analysis of surgeries at specific anatomic sites, there were no associations with NHL risk, except for a suggestive positive association for surgery involving the appendix, stomach or bowel (OR=1.24; 95% CI 0.98–1.58). When blood transfusion, anesthesia, and total number of surgeries were included in the same model, ORs for time since first transfusion and total number of surgeries remained unchanged, while the association for anesthesia weakened. These results were generally similar for both diffuse and follicular subtypes, with the exception that total number of surgeries showed a suggestive positive association with follicular (OR=1.61 for 7+ surgeries compared to 0; 95% CI 0.74–3.51) but not diffuse NHL. Conclusion: History of blood transfusion was associated with an increased risk of NHL. Total number of surgeries, type of surgery, and use of anesthesia were only weakly associated with risk, although the suggestive positive association for number of surgeries with follicular lymphoma warrants further investigation.


Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 3655-3655 ◽  
Author(s):  
James C. Gay ◽  
Timothy McCavit ◽  
David Bundy ◽  
Allison King ◽  
Harold P. Lehman ◽  
...  

Abstract BACKGROUND AND OBJECTIVE: In the Silent Cerebral Infarct Trial (SIT), regular blood transfusion therapy significantly reduced the incidence of recurrent cerebral infarctions in children with sickle cell anemia (SCA). As a follow-up analysis of the SIT Trial, we compared healthcare utilization, as measured by adverse events, hospitalizations and costs in regularly transfused children (transfusion group) to those who were not transfused (observation group). <>METHODS: In this multi-center trial, we randomly allocated 196 children aged 5-15 years with SCA and prior history of silent cerebral infarcts (SCI) to receive monthly blood transfusion or observation for at least 36 months or until a study endpoint was reached. The number of and reasons for hospitalizations were recorded at each site. The transfusion group was determined by a protocol approach, with all patients receiving regular transfusions over a period of at least 6 months included, irrespective of the original group assignment in the SIT study. Estimated costs per day of hospitalization were determined using data obtained from the 14 SIT institutions which contributed administrative data to the Pediatric Health Information System (PHIS) database maintained by the Children's Hospital Association. Inpatient costs were based on length of hospital stay, modified by the occurrence of categories of adverse events in the following non-overlapping hierarchy: acute chest syndrome, vaso-occlusive pain crisis, fever/infection, exchange transfusion, surgery and asthma. Outpatient expenses not related to transfusion or iron chelation were considered equivalent for transfused patients and controls for the purposes of this study and were not included in the costing model. Chelation and blood transfusion costs were based on a child that weighed 30 kg and received 20 mg/kg/day of deferoxamine or deferasirox. Follow-up occurred from time of random allocation to primary endpoint (overt stroke or new or progression of SCI) or exit MRI, whichever came first. The SIT Trial is registered at www.clinicaltrials.gov (NCT00072761). <> <>RESULTS: A total of 90 and 106 patients comprised the final transfusion and observation groups, respectively. Fifteen of the patients originally randomly allocated to the transfusion group crossed over to the observation group by either never receiving blood transfusion (N=9) or receiving less than 6 months of regular blood transfusion (N=6) and were counted as not being effectively transfused (i.e., part of the observation group). The mean follow up for individuals who did or did not receive blood transfusion therapy was 3.04 and 3.01 years, respectively. The average age of all participants at randomization was 10.0 years, with 43.4% males. There were 144 hospitalizations in the transfusion group and 269 in the observation group; average length of hospital stay was 2.5±1.8 days vs. 3.4±2.2 days for transfused and observation groups, respectively (p<0.001). An average of 1.6 and 2.5 hospitalizations occurred per patient with a total of 358 and 912 patient days for patients in the transfusion and observation groups, respectively. The most common reason for hospitalization was an acute pain episode (49.6%), followed by acute chest syndrome (9.4%). For every 100 children with history of SCI treated with regular blood transfusions for one year, there were 71 fewer hospital days for all SCA-related conditions per SCI prevented (157 fewer days/2.19 fewer SCIs) when compared to 100 children with SCA and history of SCIs who are not treated with transfusion therapy. Hospitalization costs were reduced 54% per year ($4,302 vs. $9,407) for children receiving blood transfusion therapy compared to those observed. Total yearly costs not related to hospitalization for patients in the transfusion group ranged from $18,149 to $67,361/year, depending on the estimated costs for the type of chelation used and type of red blood cell transfusion (manual partial exchange or apheresis). (Table) CONCLUSIONS: Children with SCA and silent cerebral infarcts receiving regular blood transfusions have a 54% relative reduction in hospitalization cost when compared to children with SCA; however their outpatient costs of monthly prophylactic blood transfusions are high and heavily dependent upon the type of blood transfusion therapy and choice of chelation therapy. Table Table. Disclosures McCavit: Novartis: Speakers Bureau; Pfizer: Consultancy, Research Funding. King:HRSA: Research Funding; NIH - NHLBI: Research Funding; NIH - BMTCTN: Research Funding. Strouse:NHLBI: Research Funding; HRSA: Consultancy; Maryland Dept of Health and Mental Hygiene: Research Funding; HRSA: Research Funding. Casella:Johns Hopkins: Patents & Royalties; Mast Therapeutics: Research Funding; ImmunArray: Patents & Royalties.


2017 ◽  
Vol 24 (1) ◽  
Author(s):  
Bobbi Arifin ◽  
Untung Tranggono

Objective: To know the factors that can predict the need for blood transfusions after transurethral resection of the prostate (TURP) at Dr. Sardjito General Hospital. Material & method: This is a retrospective study. Analysis performed on 250 patients who underwent TURP between the years 2013 to 2015. The independent variables evaluated were age, body mass index (BMI), the estimated size of the prostate by transabdominal ultrasonography (TAUS), duration of surgery, hemoglobin level (Hb), the value of international normalized ratio (INR), history of hypertension, diabetes mellitus (DM) and the use of aspirin, and leukosituria. The dependent variable evaluated was the estimated amount of bleeding which was described by the blood transfusion requirements in patients after TURP. Results: Hb levels before surgery (p=0.000), history of hypertension (p=0.001) and a history of aspirin consumption (p=0.008) had a significant association with blood transfusion after TURP. The Hb cut-off value was 12.45 g/dl. Age, BMI, the estimated size of the prostate by TAUS, duration of surgery, history of diabetes and leukosituria did not have a significant association with blood transfusion after TURP. Conclusion: The results showed that Hb levels before surgery, a history of hypertension and aspirin usage can be predictive factors for blood transfusions after TURP.


Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4282-4282
Author(s):  
Aroob Sweidan ◽  
Sowjanya Vuyyala ◽  
Peter Xie ◽  
Mohammad Alhyari ◽  
Vrushali S. Dabak ◽  
...  

Abstract Background: Sickle cell disease (SCD) patients are at risk of developing multiple complications from transfusions, including alloimmunization to red blood cell (RBC) antigens, delayed hemolytic transfusion reactions, and hyperhemolysis syndrome (HS). HS is a serious complication of transfusion characterized by the destruction of both transfused and autologous RBCs with resulting severe anemia and post transfusion hemoglobin lower than pretransfusion levels. We report the case of a middle age female patient with known SCD who developed severe HS following a blood transfusion. We aim to remind physicians of the importance of conservative blood transfusions in SCD patients in order to avoid serious transfusion-related complications. Case report: A 57-year-old African American patient, with known history of SCD who was doing well with a baseline hemoglobin (Hgb) of 6-7 g/dl. Transfusion history included 4 units of Packed Red Blood Cell (PRBC) during the 5 years prior to this presentation, all of which for mild, non-resolving vaso-occlusive pain crisis. Her most recent transfusion was 7 days prior to her presentation, she received 1 unit of PRBC for a Hgb level of 6.3 g/dl, associated with mild musculoskeletal pain and fatigue. She presented to the Emergency Department 4 days later with worsening fatigue, decreased oral intake and dark urine. On presentation, she was normotensive, afebrile and mildly tachycardic. She had increasing oxygen requirements to maintain O2 saturation above 94%. Her blood work showed a Hgb of 2.8 g/dl (12-15 g/dL), hematocrit 8.3 % (36-46 %), RBC count 0.87 M/uL (4.15-5.55 M/uL), Mean Corpuscular Volume 95.5 fl (80-100 fl), elevated White Cell Count at 28.4 K/uL (3.8-10.6 K/uL), and platelet count 125 K/uL (150-450 K/uL). Hemolysis labs showed low haptoglobin of &lt; 30 mg/dl (30-200 mg/dl), elevated Lactate Dehydrogenase at 3420 IU/L (&lt; 250 IU/L), total bilirubin 2.7 mg/dl (&lt; 1.2 mg/dl), direct bilirubin 0.6 mg/dl (0-0.3 mg/dl), and reticulocyte count 3.5% (0.5-1.5 %; reticulocytopenia relative to degree of anemia). A disseminated intravascular coagulation (DIC) panel showed fibrinogen of 263 mg/dL (200-450 mg/dL), D-dimer greater than 20 ug/mL (&lt; 0.50 ug/ml), prothrombin time of 19.8 seconds (s) (11.5-14.5 s), and partial thromboplastin time of 32 s (22-36 s). High sensitivity troponin was elevated at 650 ng/L (&lt; 19 ng/L). Antibody screen and direct antiglobulin test (DAT) were negative. Peripheral blood smear showed severe anemia with marked anisopoikilocytosis including numerous blister cells, occasional sickle cells and numerous nucleated red blood cells. The recent history of blood transfusion and the current laboratory workup were consistent with HS. Patient was admitted to the intensive care unit (ICU) for management; she initially received 1g intravenous iron dextran and intravenous immunoglobulin (IVIG) 0.4 g/kg for 5 days. She was also started on erythropoietin, folic acid, and vitamin B12. Her reticulocyte count improved to 19%. Given no improvement in Hgb levels, systemic steroids were started after ruling out infectious etiologies. She initially received methylprednisolone 125mg daily for 2 days, followed by oral prednisone 60mg daily for 7 days. Patient had increased oxygen requirements during admission, had an elevated lactate to 4 mmol/L, and had a drop in Hgb to 2.1 g/dL. She was still managed conservatively with oxygen supplementation and intravenous crystalloid fluids. The decision was to avoid transfusions unless they were life-saving. Patient remained in the ICU unit for 5 days, then was transferred to the hematology floor where she remained hospitalized for 7 days. Oxygen requirements and patient's symptoms steadily improved, hemolysis labs trended down, and reticulocyte count improved. Hgb levels improved gradually to highest of 5.7 g/dl prior to discharge. Patient was then discharged to follow up with her hematologist in the outpatient setting. Conclusion: This case aims to highlight the importance of early recognition of HS to avoid wrong management with RBC transfusion. Our patient had severe anemia and was managed with transfusion-free approach with good outcome. This case is also meant to remind physicians of the importance of conservative blood transfusions in SCD patients in order to avoid serious and life-threatening transfusion-related complications. Disclosures No relevant conflicts of interest to declare.


2012 ◽  
Vol 33 (4) ◽  
pp. E5 ◽  
Author(s):  
Efrem M. Cox ◽  
Kathleen E. Knudson ◽  
Sunil Manjila ◽  
Alan R. Cohen

The authors present the first report of spinal congenital dermal sinus with paramedian dual ostia leading to 2 intradural epidermoid cysts. This 7-year-old girl had a history of recurrent left paramedian lumbosacral subcutaneous abscesses, with no chemical or pyogenic meningitis. Admission MRI studies demonstrated bilateral lumbar dermal sinus tracts and a tethered spinal cord. At surgery to release the tethered spinal cord the authors encountered paramedian dermal sinus tracts with dual ostia, as well as 2 intradural epidermoid cysts that were not readily apparent on MRI studies. Congenital dermal sinus should be considered in the differential diagnosis of lumbar subcutaneous abscesses, even if the neurocutaneous signatures are located off the midline.


Diagnostics ◽  
2020 ◽  
Vol 11 (1) ◽  
pp. 1
Author(s):  
Maria Vlachou ◽  
Vasileios Kamperidis ◽  
Efthymia Vlachaki ◽  
Georgios Tziatzios ◽  
Despoina Pantelidou ◽  
...  

Patients with beta-thalassemia major (β-ΤΜ) may develop cardiac arrhythmias through a multifactorial mechanism. The current study evaluated the association of cardiac structure and function on echocardiography with atrial ectopic burden on 24-hour tape recording in β-ΤΜ patients. This prospective study included consecutive β-ΤΜ patients. Demographic, laboratory, echocardiographic, cardiac magnetic resonance (CMR) T2* and 24-hour tape recording data were prospectively collected. The patients were classified according to the median value of premature atrial contractions (PACs) on 24-hour tape. In total, 50 β-TM patients (37.6 ± 9.1 years old, 50% male) were divided in 2 groups; PACs ≤ 24/day and > 24/day. Patients with PACs > 24/day were treated with blood transfusion for a longer period of time (39.0 ± 8.6 vs. 32.0 ± 8.9 years, p < 0.007), compared to their counterparts. Older age (OR: 1.121, 95% CI: 1.032–1.217, p = 0.007), longer duration of blood transfusion (OR:1.101, 95% CI:1.019–1.188, p = 0.014), larger LV end-diastolic diameter (OR: 4.522, 95% CI:1.009–20.280, p = 0.049), higher values of LA peak systolic strain (OR: 0.869, 95% CI: 0.783–0.964, p = 0.008), higher MV E/E′ average (OR: 1.407, 95% CI: 1.028–1.926, p = 0.033) and higher right ventricular systolic pressure (OR: 1.147, 95% CI: 1.039–1.266, p = 0.006) were univariably associated with PACs > 24/day. LA peak systolic strain remained significantly associated with PACs > 24/day after adjusting for the duration of blood transfusions or for CMR T2*. The multivariable model including blood transfusion duration and LA peak systolic strain was the most closely associated with PACs > 24/day. Receiver operating characteristic curve analysis identified a left atrial peak systolic strain of 31.5%, as the best cut-off value (83% sensitivity, 68% specificity) for prediction of PACs > 24/day. In β-TM patients, LA peak systolic strain was associated with the atrial arrhythmia burden independently to the duration of blood transfusions and CMR T2*.


2003 ◽  
Vol 99 (2) ◽  
pp. 287-290 ◽  
Author(s):  
Celia C. D'Errico ◽  
Hamish M. Munro ◽  
Steven R. Buchman ◽  
Deborah Wagner ◽  
Karin M. Muraszko

Object. This prospective, randomized, placebo-controlled, double-blind trial was undertaken to assess the efficacy of aprotinin in reducing the need for blood transfusions in 39 children undergoing reconstructive craniofacial surgery. Methods. Two demographically similar groups—a total of 39 patients with a mean age of 1.2 ± 1.2 years—were studied. The efficacy of aprotinin (240 mg/m2 administered intravenously over 20 minutes, followed by infusions of 56 mg/m2/hr) was compared with that of an equal infusion of 0.9% saline (placebo). Patients in the aprotinin group received less blood per kilogram of body weight than patients in the placebo group (32 ± 25 ml/kg compared with 52 ± 34 ml/kg, respectively; p = 0.04). Those patients in whom aprotinin was administered experienced less change in their hematocrit levels during surgery (aprotinin −33 ± 13% compared with placebo −44 ± 9%, p = 0.01). Each patient underwent a transfusion as per study protocol, and there was no significant change in hematocrit levels from the beginning to the end of surgery. The surgical faculty judged blood loss in patients in the aprotinin group to be significantly less than usual (p = 0.03). The use of aprotinin was also associated with reduced blood transfusion requirements during the first 3 postoperative days (p = 0.03). There was no adverse event reported in either the aprotinin or placebo group. Conclusions. Aprotinin decreased blood transfusion requirements in pediatric patients undergoing craniofacial reconstruction, thereby reducing the risks associated with exposure to banked blood components.


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